Acute myeloid leukaemia with myelodysplastic features in children: a report of Japanese Paediatric Leukaemia/Lymphoma Study Group
Acute myeloid leukaemia with myelodysplastic features in children: a report of Japanese Paediatric Leukaemia/Lymphoma Study Group
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儿童急性髓性白血病伴骨髓增生异常：日本小儿白血病/淋巴瘤研究组的报告
DOI：
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发表时间：
2014
影响因子：
6.5
通讯作者：
S. Adachi
中科院分区：
文献类型：
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作者：
A. Kinoshita;H. Miyachi;H. Matsushita;M. Yabe;T. Taki;Tomoyuki Watanabe;A. Saito;D. Tomizawa;T. Taga;Hiroyuki Takahashi;H. Matsuo;K. Kodama;K. Ohki;Y. Hayashi;A. Tawa;K. Horibe;S. Adachi
儿童急性髓性白血病（AML）伴骨髓增生异常特征的临床特征和预后相关性仍有待阐明。我们前瞻性地检查了443例新诊断的儿童原发性AML多中心临床试验患者，根据2008年世界卫生组织的分类，发现93例（21.0%）为“伴有骨髓增生异常相关变化的AML”（AML-MRC），其中59例仅诊断为骨髓增生异常相关细胞遗传学，28例仅诊断为多系发育不良，6例诊断为两者联合。与111例“未另行说明的急性髓细胞白血病”（AML-NOS）患者相比，“AML-MRC”患者的发病年龄更小，白色血细胞计数更低，20-30%骨髓原始细胞的发生率更高，细胞遗传学不利，Fms样酪氨酸激酶3内部串联重复（FLT 3-ITD）、NPM 1和CEBPA突变的频率更低。“AML-MRC”患者的完全缓解率和3年无事件生存概率显著更差（分别为67.7 vs. 85.6%，P <0.01，37.1% vs. 53.8%，P = 0.02），但3年总生存率和无复发生存率与“AML-NOS”患者相当。通过多变量分析，FLT 3 ‐ITD仅与总生存期较差相关。这些结果支持即使在儿童中也存在“AML‐MRC”类别的独特特征。
The clinical characteristics and prognostic relevance of acute myeloid leukaemia (AML) with myelodysplastic features remains to be clarified in children. We prospectively examined 443 newly diagnosed patients in a multicentre clinical trial for paediatric de novo AML, and found ‘AML with myelodysplasia‐related changes’ (AML‐MRC) according to the 2008 World Health Organization classification in 93 (21·0%), in whom 59 were diagnosed from myelodysplasia‐related cytogenetics alone, 28 from multilineage dysplasia alone and six from a combination of both. Compared with 111 patients with ‘AML, not otherwise specified’ (AML‐NOS), patients with ‘AML‐MRC’ presented at a younger age, with a lower white blood cell count, higher incidence of 20–30% bone marrow blasts, unfavourable cytogenetics and a lower frequency of Fms‐like tyrosine kinase 3 internal tandem duplication (FLT3‐ITD), NPM1 and CEBPA mutations. Complete remission rate and 3‐year probability of event‐free survival were significantly worse in ‘AML‐MRC’ patients (67·7 vs. 85·6%, P < 0·01, 37·1% vs. 53·8%, P = 0·02, respectively), but 3‐year overall survival and relapse‐free survival were comparable with ‘AML‐NOS’ patients. By multivariate analysis, FLT3‐ITD was solely associated with worse overall survival. These results support the distinctive features of the category ‘AML‐MRC’ even in children.
分析免疫表型分析在预测急性白血病临床结果方面的效用的新方法。
影响因子：
11.4
作者：
Paietta,E;Andersen,J;Wiernik,PH
通讯作者：
Wiernik,PH
336 例新发急性髓系白血病患者的骨髓生成障碍的评估：粒细胞生成障碍对于缓解和生存的重要意义。
影响因子：
11.4
作者：
Goasguen,JE;Matsuo,T;Cox,C;Bennett,JM
通讯作者：
Bennett,JM